Antithrombin III Deficiency

Common Name(s)

Antithrombin III Deficiency

Deficiency of antithrombin III is a major risk factor for venous thromboembolic disease. Two categories of AT-III deficiency have been defined on the basis of AT-III antigen levels in the plasma of affected individuals. The majority of AT-III deficiency families belong in the type I (classic) deficiency group and have a quantitatively abnormal phenotype in which antigen and heparin cofactor levels are both reduced to about 50% of normal. The second category of AT-III deficiency has been termed type II (functional) deficiency. Affected individuals from these kindreds produce dysfunctional AT-III molecules; they have reduced heparin cofactor activity levels (about 50% of normal) but levels of AT-III antigen are often normal or nearly normal (summary by {5:Bock and Prochownik, 1987}). The 2 categories of antithrombmin III deficiency have been classified further. Type I (low functional and immunologic antithrombin) has been subdivided into subtype Ia (reduced levels of normal antithrombin), and type Ib (reduced levels of antithrombin and the presence of low levels of a variant). Type II (low functional but normal immunologic antithrombin) has been subdivided into subtype IIa (functional abnormalities affecting both the reactive site and the heparin-binding site of AT3); subtype IIb (functional abnormalities limited to the reactive site); and subtype IIc (functional abnormalities limited to the heparin-binding site) (summary by {15:Lane et al., 1992}).
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Antithrombin III Deficiency" for support, advocacy or research.

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Antithrombin III Deficiency" returned 35 free, full-text research articles on human participants. First 3 results:

Portal vein thrombosis after aortic valve replacement surgery in a patient with antithrombin III deficiency--case presentation.
 

Author(s): Yu-qing Wang, Qiu-lin Chen, Da Zhu, Li Dong

Journal:

 

We presented an unique case of portal vein thrombosis (PVT) after aortic valve replacement due to antithrombin III (ATIII) deficiency. PVT after aortic valve replacement (AVR) is a serious complication, which has not previously been reported.

Last Updated: 3 Jun 2014

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Surgical treatment of atrial septal defect in a patient with familial antithrombin-III deficiency.
 

Author(s): Tsutomu Hattori, Mitsumasa Hata, Akira Sezai, Shinji Wakui, Motomi Shiono

Journal: Ann Thorac Cardiovasc Surg. 2013 ;19(4):323-5.

 

A 41-year-old woman with familial antithrombin-III deficiency was admitted to our hospital for patch closure of an atrial septal defect. Antithrombin-III activity was 43% and its antigen level was 12.2 mg/dl, was diagnosed so type I antithrombin-III deficiency was diagnosed.

Last Updated: 22 Aug 2013

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Multiple isolated sinus dural arteriovenous fistulas associated with antithrombin III deficiency--case report--.
 

Author(s): Atsushi Saito, Noboru Takahashi, Yuichi Furuno, Hironaga Kamiyama, Shinjitsu Nishimura, Hiroshi Midorikawa, Michiharu Nishijima

Journal: Neurol. Med. Chir. (Tokyo). 2008 Oct;48(10):455-9.

 

A 55-year-old man presented with a rare case of multiple isolated sinus dural arteriovenous fistulas (AVFs) associated with antithrombin (AT) III deficiency manifesting as sudden onset of headache and gait disturbance. Increased arterial shunting flow had caused intraventricular hemorrhage ...

Last Updated: 24 Oct 2008

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Antithrombin III Deficiency" returned 2 free, full-text review articles on human participants. First 3 results:

Plasma antithrombin III deficiency in ischaemic stroke in the young.
 

Author(s): D Nagaraja, R Christopher, M Tripathi

Journal: Neurol India. 1999 Jun;47(2):155-6.

 

A deficiency of plasma antithrombin III has been identified as a potential risk factor for thrombosis. In a pilot study of 56 patients aged less than 40 years who presented with ischaemic stroke of unknown etiology, we detected only one case of plasma antithrombin III deficiency. ...

Last Updated: 11 Aug 1999

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Catastrophic secondary antiphospholipid syndrome with concomitant antithrombin III deficiency.
 

Author(s): V R Peddi, K S Kant

Journal: J. Am. Soc. Nephrol.. 1995 May;5(11):1882-7.

 

The association between thrombotic events and primary or secondary antiphospholipid/anticardiolipin syndrome is now well recognized. A spectrum of renal involvement ranging from glomerular thrombosis to renal infarction has been described. A case of systemic lupus erythematosus with ...

Last Updated: 30 Aug 1995

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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Safety, Pharmacokinetics and Efficacy of an AT-III Concentrate.
 

Status: Recruiting

Condition Summary: Antithrombin III Deficiency

 

Last Updated: 17 Feb 2014

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Last Updated: 21 Jul 2014

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